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| Year:
2017 |
Month:
January
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Volume:
6 |
Issue:
1 |
Page:
IO01 - IO05 |
Hemophagocytic Lymphohistiocytosis in Adults and Adolescents - Experience from a Tertiary Care Centre in South India
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Correspondence
Shailendra Prasad Verma, Rakesh Naik V, Debdatta Basu, Kolar Vishwanath Vinod, Rakhee Kar, Tarun Kumar Dutta,
Dr. Shailendra Prasad Verma,
Flat Number 506, Teachers Flat, T.G. Campus, Khadra, Lucknow-226020, Uttar Pradesh, India.
E-mail: drspkgmu@rediffmail.com :
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Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of immune dysregulation. It is characterized by hypercytokinemia and macrophage activation resulting in fever, cytopenia, splenomegaly and hyperferritinemia leading to fatal outcomes if untreated.
Aim: To study clinical profile, diagnostic and etiological workup, treatment and prognosis of hemophagocytic lymphohistiocytosis patients.
Materials and Methods: We report retrospective analysis of 8 cases of adult and adolescent HLH admitted over a period of 2 years at JIPMER, a tertiary care centre in South India.
Results: Mean age of patients was 27 year (range 13 to 57 years) and 3 were adolescents. Median duration of symptoms was 10 days (5-60 days). Common presenting symptoms were fever, jaundice, abdominal pain, rash and seizures. Physical findings included pallor, icterus, splenomegaly, hepatomegaly and lymphadenopathy. Laboratory findings were variable cytopenia with pancytopenia in 65% cases, hyperferritinemia (100%), hypertriglyceridimia (75%) and elevated serum bilirubin (62.5%) and liver enzymes (87.5%). Underlying cause could be detected in only 6 patients with one each of dengue fever, lymphoma, tuberculosis, EBV infection, scrub typhus and juvenile idiopathic arthritis. Median overall survival at the end of 1.5 years was 62%.
Conclusion: Low threshold for suspicion, adequate evaluation and timely treatment can improve outcome in patients of HLH.
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